Titlebook: Cytokine Storm Syndrome; Randy Q. Cron,Edward M. Behrens Book 20191st edition Springer Nature Switzerland AG 2019 Hemophagocytic lymphohis

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Transnational Threat in the ‘Globalized’ Erae), the laboratory findings associated with these diseases, and the currently recommended diagnostic strategies. The pathogenesis of primary forms of hemophagocytic lymphohistiocytosis (HLH) is based on an impaired function of cytotoxic T-lymphocytes and NK cells, which then leads to an inability of

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Oedema and Increased Vascular Permeabilityy CD8) and hemophagocytic macrophages producing proinflammatory cytokines. MAS has been reported in association with almost every rheumatic disease, but it is by far the most common in systemic juvenile idiopathic arthritis (sJIA). Clinically, MAS is similar to familial or primary hemophagocytic lym

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Treatment of ANCA-Associated Vasculitidesed for release of perforin-containing cytotoxic granule constituent. Perforin is expressed by subsets of CD8. T cells and NK cells, representing lymphocytes that share mechanism of target cell killing yet display distinct modes of target cell recognition. Here, we highlight recent findings concernin

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Rare Diseases of the Immune Systemrticular interest in primary hemophagocytic lymphohistiocytosis (pHLH) since all of the genetic defects associated with this disorder cause diminished cytotoxic capacity of NK cells and T lymphocytes, and assays of NK cell killing are used clinically for the diagnosis of HLH. Herein, we review human

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https://doi.org/10.1007/978-1-349-19222-9immune functions in a wide variety of ways. The relevant cytokines for each CSS are likely a result of differing combinations of environmental triggers and host susceptibilities. Because cytokines or their receptors may be specifically targeted by biologic therapeutics, understanding which cytokines

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The Late Twenties and Early Thirtiesns. Whether the result of a genetic lesion that causes defective granule-dependent cytotoxicity, excessive lymphoproliferation, or an overwhelming infection representing a unique antigenic challenge, PIDs can display a proclivity for cytokine storm syndrome (CSS) development. This chapter provides a